Pheochromocytoma is a curable, rare cause of hypertension, characterized by symptoms and signs related to increased catecholamine secretion. Pregnancy and labour increase the risk of hypertensive crisis. However, antepartum diagnosis reduces both maternal and foetal mortality, allowing for safe cesarean section and resection of tumor. Control of hypertension with alpha blockers and beta blockers is the medical treatment. Surgical removal of the tumour is the definitive treatment. Hypertensive crisis needs to be treated aggressively. We report this case for the rare presentation of pheochromocytoma presenting as hypertension in pregnancy.
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Front Endocrinol (Lausanne)
December 2024
One Health Research Group, Universidad de las Americas, Quito, Ecuador.
Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors derived from chromaffin cells, with 80-85% originating in the adrenal medulla and 15-20% from extra-adrenal chromaffin tissues (paragangliomas). Approximately 30-40% of PPGLs have a hereditary component, making them one of the most genetically predisposed tumor types. Recent advances in genetic research have classified PPGLs into three molecular clusters: pseudohypoxia-related, kinase-signaling, and -signaling pathway variants.
View Article and Find Full Text PDFJCEM Case Rep
January 2025
Department of Endocrinology and Metabolism, Kanazawa University Graduate School of Medical Sciences, Kanazawa University, Kanazawa, 920-8641, Ishikawa, Japan.
Pheochromocytoma and paraganglioma (PPGL) are rare chromaffin-cell tumors producing adrenaline and/or noradrenaline, or solely dopamine. A 52-year-old man presenting with hypertension (141/79 mm Hg) and weight loss (10 kg in 6 months) was admitted to our hospital. Computed tomography revealed a massive right adrenal mass (150 mm) with partial necrosis, accompanied by multiple liver nodules.
View Article and Find Full Text PDFJ Minim Access Surg
December 2024
Department of Surgical Disciplines, All India Institute of Medical Sciences, New Delhi, India.
Introduction: Laparoscopic transperitoneal adrenalectomy was first described by Gagner M et al. Here, we present our experience of more than two decades of laparoscopic adrenalectomy performed in a single surgical unit at a tertiary care centre.
Patients And Methods: A prospectively collected database of patients undergoing laparoscopic adrenalectomy from December 1994 to May 2020 was analysed retrospectively.
Cancer Metab
December 2024
Department of Biochemistry and Molecular Biology, Mayo Clinic College of Medicine and Science, 200 First St. SW, Rochester, MN, 55905, USA.
Heterozygosity for loss-of-function alleles of the genes encoding the four subunits of succinate dehydrogenase (SDHA, SDHB, SDHC, SDHD), as well as the SDHAF2 assembly factor predispose affected individuals to pheochromocytoma and paraganglioma (PPGL), two rare neuroendocrine tumors that arise from neural crest-derived paraganglia. Tumorigenesis results from loss of the remaining functional SDHx gene copy, leading to a cell with no functional SDH and a defective tricarboxylic acid (TCA) cycle. It is believed that the subsequent accumulation of succinate competitively inhibits multiple dioxygenase enzymes that normally suppress hypoxic signaling and demethylate histones and DNA, ultimately leading to increased expression of genes involved in angiogenesis and cell proliferation.
View Article and Find Full Text PDFJ Cardiothorac Surg
December 2024
Zhejiang Taizhou Hospital, Taizhou, China.
Pheochromocytoma is rare in clinical practice, with patients typically presenting with headache, high blood pressure and sweating. Patients who develop a pheochromocytoma crisis are particularly rare. This report describes the case of a patient in a pheochromocytoma crisis who presented with severe cardiogenic shock, acute respiratory failure, and acute coronary syndrome.
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